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Developmental abnormalities

Embryogenesis defects are the basis of abnormalities in the development of the nose and paranasal sinuses. They are due to the presence of a pathological mutant gene in the genotype, as well as the influence of various harmful factors (infections, alcoholism, tobacco abuse and other intoxications affecting the genetic code). Harmful factors are especially dangerous in the second month of embryonic life, when the laying of facial elements occurs, including nose.

Often there are combined defects of the maxillofacial region, because 1st and 2nd branchial arches take part in their genesis. This also explains the immune deficiency in patients with this pathology, associated with the underdevelopment of gl. thymus (Kotov G.A., 1987), also originating from the 2nd branchial arch.

The severity of birth defects is determined by the expressiveness of the pathological gene (Efroimson V.P., 1968) and is clinically manifested as deformity, anomalies and developmental variations. So, the more pronounced the defect of an organ is, the more right it must be attributed to ugliness; weak degrees of disfigurement should be considered anomalies, and underdeveloped deviations - variations (KhilovK.L., 1960). In adults, birth defects are rare, as in most cases they are eliminated in childhood.

B.V. Shevrygin (1996) in his extensive classification of nasal defects and abnormalities, developed jointly with M.K.Manyuk, gives the following groups of congenital defects of the nose: agenesis - complete underdevelopment of the organ; hypergenesis - excessive development; hypogenesis - underdevelopment, dystopia - violation of the normal position of the body; dysgenesis - its improper development; persistence - the preservation of embryonic structures that normally disappear by a certain period of development.

Of the individual malformations of the nose, humpiness, saddle nose, obliquity, severe deformations of the nasal septum, hyperplasia, hypoplasia, and deformities of the nasal concha, which can be attributed to less pronounced defects - developmental variations, are most common.

Anomalies may include median embryonic nasal clefts, which, if expressed, can be considered as deformities. An example of such an abnormality of the nose is the fistula of the nasal dorsum, which has the appearance of an open gutter or a blind canal, from which curdled masses and desquamating epithelium stand out.

Clinically dangerous type of anomalies of the middle nasal slit is the fistulous opening in the region of the sieve plate, which leads to the formation of a brain hernia simulating a nasal polyp, or to nasal cerebrospinal fluid, which can manifest itself in adulthood. A congenital defect in the form of a splitting of the tip of the nose also belongs to this group of anomalies. As a result of the non-fusion of two plates of the anterior part of the nasal septum, both halves of it are separated by a small recess. Such a birth defect is known in the literature as “mastiff nose”. In cases where splitting of the nose develops in depth, both halves of the nose are located at a considerable distance from each other. Each half of the nose with such a birth defect, which can already be called ugliness, has its own nasal septum, and the gap between them occupies a noticeable depression, which does not have any details of the nose. Due to the separation of both halves of the nose, the facial skull expands, the eyes are located far apart. This type of ugliness is called gargolism (by the name of the mythical ugly creature - the jargon of Gargona).
It is interesting to note that the Gargon jellyfish is one of the sculptural decorations of medieval cathedrals of Gothic architecture, including the decorations of the famous Paris Notre Dame Cathedral, and the hero of the novel by V. Hugo “Notre Dame de Paris” was a freak suffering from gargolism.

A fairly common form of combined defects within the competence of maxillofacial surgeons and rhinologists is the splitting of the hard and soft palate (cleft palate). This pathology is often combined with disorders in the outer nose and, in many cases, with cleavage of the upper lip (cleft lip).

Anomalies such as congenital anterior and posterior atresias (infestations) also belong to malformations of the nose that violate its most important function, respiration. They can be one-sided and two-sided, complete and incomplete.

The anterior atresia is localized in the vestibule and the initial part of the nose, the posterior atresia in the choan. Congenital atresia is extremely rare and more often acquired. Anterior atresias are connective tissue membranes with a small hole in the middle. Posterior choanal atresia is more often bone and total. Hoan growth can be extranasal (nasopharyngeal), intranasal (located in the deep sections of the nose, anterior to the choan) and marginal (capturing directly the choan region). Bilateral atresia of the nasal cavity is particularly difficult for infants. Turning off nasal breathing disrupts the feeding of the child, who cannot combine sucking movements with breathing, and he has to be fed from a spoon.

Anomalies of the sinuses are less common. With a thorough x-ray examination, various anatomical abnormalities of the maxillary sinus are found in approximately 13% of patients. Among them, aplasia or sinus hyperplasia, the presence in them of full or partial septa located in the sagittal, oblique or horizontal direction (Dainyak L. B., 1994).

Fibrous osteodysplasia and deforming osteodystrophy (B. Paget) of individual bones of the face and nose, which can be clinically manifested at the age of 15-20 years, are referred to defects in the embryonic development of bone tissue. The main symptom of such a pathology is a slowly increasing deformation of the bony facial skull in the area of ​​the affected bone (sinus), which violates the goodness of the face (Fig. 2.4.1). In advanced cases, such a deformity, at the suggestion of R. Virchow, was called leontiasis ossea - “bone lioness” (not to be confused with the term “facies leonies” - a lion's face that is applicable to a patient with leprosy. Progression of the pathological process as a result of compression of the bone canals , in which sensitive nerves pass, may be accompanied by a headache, often very painful, poorly responding to drug therapy.The only method of treating such a pathology is surgery with a thorough nym removal of all bone changes. Operation in advanced cases is not always technically feasible.

Treatment of other congenital defects of the nose is also surgical, the methods of which and the timing of the intervention are individual and determined depending on the nature and size of the deformity. They are cosmetic and functional.
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Developmental abnormalities

  1. THROAT DEVELOPMENT ANOMALIES
    Anomalies in the development of the pharynx are quite rare, no more than 1% among all its diseases. First of all, this is the non-closure of the soft palate, which leads to a violation of the function of swallowing (ingestion of food and liquids in the nasopharynx and nasal cavity) and the function of speech - open nasal. On examination, the sagittal cleft of the soft palate in the middle of it is determined, often the uvula is absent or vice versa
  2. Anomalies in the development of the nervous system
    The intrauterine development of a person has a clear sequence and can often be disrupted by the influence of adverse factors affecting the mother's body. These factors include: • Viral diseases of the mother during pregnancy. • Insufficiency of utero-placental circulation. • Ionizing radiation. • Vibration. •
  3. Ear malformations
    Anomalies in the development of the auricle are relatively rare. All deformities of the auricles can be divided into two groups: congenital and acquired as a result of trauma. Congenital malformations are determined immediately due to cosmetic defects - excessive size (macrotia), reduced size of the auricle (microtia) or distance from the head (bulging) of the auricles. These defects
  4. Congenital anomalies and developmental defects
    Malformations are persistent morphological changes in the organ or organism as a whole, which go beyond the limits of the norm and arise in utero as a result of a violation of the development of the embryo or fetus, sometimes after the birth of a child due to a violation of the further formation of organs. These changes cause impaired functions. Synonyms of the term "malformations"
  5. Bone abnormalities
    The skeleton system serves as the site for the development of an extremely diverse circulatory, inflammatory, tumor, metabolic and congenital disorder. Indeed, the difficulties of growth, development, self-maintenance of this system and its relationship with other organs make the skeletal system unusually vulnerable to adverse effects. No wonder primary and secondary bone diseases
  6. Anomalies in the development of the nose
    Congenital anomalies of the external nose due to impaired embryonic development are relatively rare: these are the absence or insufficient development of the nose, the excessive growth of its parts, the abnormal location and development of the entire nose or its departments. In practice, such congenital malformations as a double nose, splitting of the nose, its formation in the form of one trunk or two
  7. CONGENITAL ANOMALIES OF NERVOUS SYSTEM DEVELOPMENT
    The group of diseases, which are based on congenital defects in the development of the nervous system, includes such a pathology as the Klippel-Feil anomaly, characterized by isolated and multiple synostoses of the cervical vertebrae, cranial hernia, craniostenosis (premature closure of cranial sutures), microcephaly (reduction in size skull and brain, accompanied by mental
  8. Operations for abnormalities of the uterus
    Operations with abnormalities in the development of the uterus (rudimentary horn of the uterus, two-horned uterus, double uterus) are most often performed with impaired reproductive function (infertility, miscarriage) caused by this pathology. Technique of execution. The rudimentary horn of the uterus is often detected during a developing (or interrupted) pregnancy in it. The rudimentary uterine horn is excised with attachment
  9. Genital malformations
    Definition of a concept. Congenital disorders of the anatomical structure of the genitals (VNASH) due to incomplete organogenesis are commonly referred to abnormalities in the development of the genital organs of the female body. VNASG are manifested in violation of the size, shape, proportions, symmetry, topography, and sometimes the absence (private or full) of the genitals. Frequency. According to WHO data for 1967, the frequency of VNASG was 1 per
  10. EAR DEVELOPMENT ANOMALIES
    Congenital malformations of the auricle are visually determined by cosmetic imperfections - macrotia (increase in size), microtia (decrease in size) and protrusion of the auricles. These defects are corrected using operations. With protruding ears, an oval skin flap is removed from the behind-the-ear fold. During suturing, wounds pull the auricle to the wound skin of the mastoid process.
  11. Anomalies in the development of female genital organs
    Embryonic development of the genital organs occurs in close relationship with the development of the urinary tract and kidneys. Therefore, developmental anomalies of these two systems are often found simultaneously. The kidneys develop in stages: the pre-kidney (head kidney), the primary kidney (wolf body) and the final kidney. All these formations come from nephrogenic cords located along the spine. Predpochka
  12. ANOMALIES OF DEVELOPMENT AND CONGENITAL FAULTS OF THE NERVOUS SYSTEM
    J.R. DeLong, R. D. Adame (C. R. DeLong, RDAdams) In this chapter we will focus on diseases caused by injuries or malformations of the nervous system that arose in the process of its formation, but which also have an adverse effect in adults . As a result, difficulties arise in their diagnosis and treatment of patients with which general practitioners deal and
  13. Surgery for anomalies in the development of female genital organs
    Operations on the female genital organs in connection with abnormalities of their development can be divided into the following groups: • operations on the external genital organs, hymen and vagina; • surgery for aplasia of the vagina; • operations with malformations of the uterus and
  14. Anomalies in the development and disease of the retina and optic nerve
    Retinal anomalies occur in combination with albinism, nystagmus. Inflammatory processes in the retina occur when the choroid is damaged in the form of chorioretinitis against tuberculosis, toxoplasmosis, rheumatism, etc. In children, retinal diseases are caused by general pathology and occur in the form of renal, diabetic retinopathies. It should highlight retinopathy of prematurity of varying degrees of development.
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